A Protocol Based Treatment for Debilitating Fibrosing Skin Disorders With (Anti-CD 20), Rituximab, Evaluating Safety and Efficacy

Inclusion Criteria: * (Fe) male \>, = 18 years * Fibrosing skin disorder, not fulfilling the ACR criteria for diffuse SSc * Inadequate response to methotrexate (at least 12 weeks 10 mg/w, except if not tolerated or contra-indicated * Debilitating disease defined by either one of the following: * Restriction of mobility * Disfiguration: eg: facial involvement * Severe Internal Organ involvement * Contraception for women with childbearing potential. Sexual abstinence is an alternative to contraception. Exclusion Criteria: * FVC\<, = 50% * LVEF\<, = 40% of predicted value, * DLCO\<, = 40% of predicted value * Exclusion criteria as specifically described in the protocol for anti-CD-20: * Lack of peripheral venous access. * Pregnancy or breast feeding. * Significant cardiac or pulmonary disease (including obstructive pulmonary disease). * Evidence of significant uncontrolled concomitant disease such as, but not limited to, nervous system, renal, hepatic, endocrine or gastrointestinal disorders which, in the investigator's opinion, would preclude patient participation. * Primary or secondary immunodeficiency (history of, or currently active), including known history of HIV infection. * Known active infection of any kind (excluding fungal infections of mail beds), or any major episode of infection requiring hospitalization or treatment with i.v. anti-infectives within 4 weeks of baseline or completion of oral anti-infectives within 2 weeks prior to baseline. * History of deep space/tissue infection (e.g. fasciitis, abscess, osteomyelitis) within 52 weeks prior to baseline. * History of serious recurrent or chronic infection (for screening for a chest infection a chest radiograph will be performed at screening if not performed within 12 weeks prior to screening). * History of cancer, including solid tumors, hematologic malignancies and carcinoma in situ (except basal cell and squamous cell carcinoma of the skin that have been excised and cured). * History of a severe allergic or anaphylactic reaction to a biologic agent or known hypersensitivity to any component of rituximab or to murine proteins. * Concurrent treatment with any biologic agent or DMARD other than MTX. Treatment must be discontinued 14 days prior to baseline , except for the following: azathioprine for ≥ 28 days; leflunomide for ≥ 8 weeks (or ≥ 14 days after 11 days of standard cholestyramine or activated charcoal washout); infliximab ≥ 8 weeks; adalimumab ≥ weeks. * Previous treatment with \> 1 biological agent. * Treatment with any investigational agent within 28 days of baseline or 5 half-lives of the investigational drug (which ever is the longer). * Receipt of any vaccine within 28 days prior to baseline * Intolerance or contraindications to i.v. glucocorticoids. * Positive serum human chorionic gonadotropin (hCG) measured at screening or a positive pregnancy test prior to the first rituximab infusion. * Positive tests for hepatitis B surface antigen (HBsAg), hepatitis B core antibody (HBcAb) or hepatitis C serology. * Hemoglobin \< 8.0 g/dL. * Concentrations of serum IgG and/or IgM below 5.0 and 0.40 mg/mL, respectively. * Absolute neutrophil count (ANC) \< 1.5 X 10³/µL.